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[Pretherapeutic diagnosis of fibrous dysplasia].

A safe differentiation of fibrodysplastic lesions from "real" bone tumours is of high importance because a fibrous dysplasia often requires no further therapy. While polyostotic involvement of fibrous dysplasia can be safely diagnosed before therapy, in monostotic disease differential diagnostic problems may occur. In the present investigation only in 6 of 14 mon- and biostotic lesions caused by fibrous dysplasia a correct diagnosis could be established by radiologic methods. However, in all cases of fibrous dysplasia malignancy could be excluded by radiology and the false diagnosis had no therapeutic consequences.

Adolescent↗

Fibrous dysplasia with degenerative atypia: a benign lesion potentially mistaken for sarcoma.

Fibrous dysplasia is a benign disorder of bone in which proliferating fibrous tissue replaces the bony spongiosa. Cytologic atypia is generally not considered a feature of this proliferating tissue. We present a case of fibrous dysplasia with marked atypical nuclear changes consistent with degenerative or regressive changes. A 42-year-old man presented with an osteolytic lesion of the right iliac bone. Histologic study demonstrated a fibro-osseous lesion with woven bone trabeculae and bland-looking fibrous tissue. Several areas showed atypical cells with enlarged pleomorphic nuclei and bizarre features. There was no change in the nuclear-cytoplasmic ratio nor were mitotic figures identified. The differential diagnosis is discussed. When radiographic and other histologic findings suggest fibrous dysplasia, the atypical nuclear changes should not, by themselves, alter the diagnosis.

Adult↗

[Cryogenic action in fibrous dysplasia of bony tissue].

The author shares his experience with cryosurgical treatment of fibrous dysplasia of the bone tissue. The operative methods described which use local freezing of the residual bone cavities allowed to improve the outcomes and to decrease the amount of recurrent diseases.

Adult↗

Adamantinoma of tibia with predominant features of fibrous dysplasia--a case report.

We report a case of adamantinoma of the tibia resembling fibrous dysplasia. The patient was a 55-year-old male, and complained of pain in the right lower leg. Roentgenographs showed a well demarcated osteolytic lesion with small foci of calcification and septation within the diaphysis of the distal tibia. The cortex was partially disrupted. Histologically, initial biopsy specimen showed fibrous connective tissue and trabeculae of immature woven bone, strongly suggestive of fibrous dysplasia. The lesion recurred and the second biopsy revealed nests of spindle cells and tubular epithelial structures embedded in granulation type-fibrous tissue. Immunohistochemically, both the nests of spindle cells and the tubular structures gave a positive reaction for cytokeratin. The present case emphasizes once again that histological diagnosis of fibrous dysplasia of the tibia should be made carefully with exclusion of the possibility of adamantinoma.

Ameloblastoma↗

[An unusual association: Jaffe-Linchtenstein syndrome, arcuate ligament syndrome and hepatic adenoma].

A case of a woman affected by an unusual association of rare diseases, is presented. The patient was referred to our department for acute anaemia. Preoperative investigations revealed that the patient was affected by fibrous polyostotic dysplasia, so called Jaffè-Lichtenstein syndrome. The presence of skin brown spot and endocrine disorders requiring pill administration, allows to classify the patient as carrier of Albright syndrome. Moreover, the angiography pointed out a celiac trunc stenosis (Dunbar syndrome). The long-standing administration of the pill could be the cause of bleeding adenoma. The patient underwent hepatic resection. We did not treat the Dunbar syndrome because of poor symptoms. From literature, we review some opinions on the fibrous dysplasia of the bone.

Adenoma↗

Adamantinoma of long bone. An analysis of nine new cases with emphasis on metastasizing lesions and fibrous dysplasia-like changes.

Nine new cases of adamantinoma of long bone are presented. The age range, location, and symptomatology are similar to those in precious reports. Histologically most of the tumors have a "basaloid" or "spindled" pattern. A squamoid pattern with keratinization or an "angioblastic" pattern is seen less frequently. In all cases with adequate material there has been an associated frbrous dysplasia-like lesion is interpreted as being a part of the spectrum of mesenchymal differentiation of which the tumor is capable. Two cases in which metastasis occurred are compared to previously reported metastasizing cases. Few criteria are available that identify the potentially metastasizing tumor.

Adolescent↗

Primary musculoskeletal tumors of fibrous origin.

Tumors of fibrous origin include fibrous dysplasia (FD), fibroxanthoma (nonossifying fibroma), cortical desmoid, desmoplastic fibroma, fibrosarcoma, and malignant fibrous histiocytosis (MFH). Benign fibrous lesions (FD, fibroxanthoma, and cortical desmoid) frequently demonstrate pathognomonic radiologic characteristics obviating the need for biopsy. Indeed, biopsy of these lesions can occasionally lead to confusion with more aggressive lesions. Desmoplastic fibroma and the malignant fibrous lesions (fibrosarcoma and MFH) often reveal nonspecific imaging features of a solitary nonmineralized lesion with aggressive characteristics. However, imaging is important as with other neoplasms in delineating the extent of involvement for staging purposes. This article reviews the spectrum of clinical characteristics, pathology, imaging appearances, treatment, and prognosis of lesions of fibrous origin in bone.

Bone Diseases↗

Fibrous dysplasia. Pathophysiology, evaluation, and treatment.

Fibrous dysplasia is a common benign skeletal lesion that may involve one bone (monostotic) or multiple bones (polyostotic) and occurs throughout the skeleton with a predilection for the long bones, ribs, and craniofacial bones. The etiology of fibrous dysplasia has been linked to an activating mutation in the gene that encodes the alpha subunit of stimulatory G protein (G(s)alpha) located at 20q13.2-13.3. Most lesions are monostotic, asymptomatic, and identified incidentally and can be treated with clinical observation and patient education. Bisphosphonate therapy may help to improve function, decrease pain, and lower fracture risk in appropriately selected patients with fibrous dysplasia. Surgery is indicated for confirmatory biopsy, correction of deformity, prevention of pathologic fracture, and/or eradication of symptomatic lesions. The use of cortical grafts is preferred over cancellous grafts or bone-graft substitutes because of the superior physical qualities of remodeled cortical bone.

Bone Transplantation↗

Juvenile intracortical adamantinoma of the tibia with predominant osteofibrous dysplasia-like features.

In view of the still disputed relationship between adult adamantinoma and osteofibrous dysplasia in children, a unique case of adamantinoma, indicating a direct relationship between the two lesions, is presented with a review of the literature. The patient was a six-year-old boy who complained of pain and swelling in the left lower leg. Roentgenographs showed a loculate osteolysis surrounded by sclerosis within the cortex of the tibial shaft that would be typical of osteofibrous dysplasia. Although an osteofibrous-dysplastic component predominated histologically, some small islands of epithelial cells were scattered throughout the lesion. Immunohistochemically, the tumor cells of these epithelial islands gave a constant positive reaction for cytokeratin as well as vimentin, while the stromal cells in the osteofibrous dysplasia-like lesion were positive for vimentin only. This type of lesion is recorded in the Bone Tumor Registry of Westphalia at a rate of 8.3% for osteofibrous dysplasia, and of 25% for adamantinoma. A review of the literature, yielding reports with remarkable uniformity on 14 cases beyond the present one, suggests the existence of a separate clinicopathologic entity to be called juvenile intracortical adamantinoma with predominant osteofibrous dysplasia-like features, and which might be a regressing form of adamantinoma specific in childhood.

Ameloblastoma↗

Treatment of craniomaxillofacial fibrous dysplasia: how early and how extensive?

Twenty-eight craniomaxillofacial fibrous dysplasia patients were treated as early as the symptoms occurred. The principles of surgical treatment were based on the zones of involvement: total excision of dysplastic bone of fronto-orbital, zygoma, and upper maxillary origin (zone 1) and bone reconstruction primarily; conservative excision on hair-bearing skull (zone 2), central cranial base (zone 3), and tooth-bearing bones (zone 4); and optic canal decompression on patients with orbital dysplasia and decreasing visual acuity. Patients were followed for 1 to 11 years (average 5.3 years). No recurrence or invasion of the fibrous dysplasia into the grafted bone was seen. One patient had orthognathic maxillary osteotomy on the reconstructed maxilla 6 years after initial reconstruction. Five of 19 patients with alveolar dysplasia had a recurrence and were reshaped. One patient had mandibular sagittal osteotomies to set back the prognathic, fibrous dysplasic mandible after three attempts at conservative shaving. Another patient with mandibular fibrous dysplasia had recurrence with pain and a hemimandibulectomy with successful immediate free vascularized iliac bone graft reconstruction.

Adolescent↗

Surgical outcomes using bioabsorbable plating systems in pediatric craniofacial surgery.

The purpose of this study was to evaluate the surgical outcomes of the 1.5-mm LactoSorb plating system (Walter Lorenz Surgical, Inc., Jacksonville, FL, U.S.A.) used to stabilize the osteotomized calvarial bone in pediatric patients who have undergone craniofacial surgery. The records of 33 consecutive pediatric patients who underwent craniofacial surgery from January 1997 through December 1997 were reviewed. There were 18 male and 15 female patients, and the age ranged from 4 months to 12 years. Patients were followed-up at 1 week, 1 month, 3 months, 6 months, and 12 months after surgery. For those patients reviewed, the following information is included: age, sex, diagnosis, surgical procedures, number and size of LactoSorb plates and screws used in each patient, operative difficulty of the screws and the heat pack, and postoperative complications, including wound healing, palpability, and infection. The LactoSorb plating system was used to stabilize the osteotomized calvarial bones in 33 patients who were diagnosed with: 1) craniosynostosis, 2) hydrocephalus, 3) fibrous dysplasia, or 4) cranial deformation. Orbital rim advancement and anterior cranial vault reshaping were performed in 17 patients. Posterior cranial vault reshaping, orbital rim advancement, and anterior cranial vault reshaping were performed in eight patients. Posterior cranial vault reshaping only was performed in seven patients. Excision of fibrous dysplasia from temporal bone was performed in one patient. One patient had a postoperative wound infection, and LactoSorb plates were palpable postoperatively in four patients. The LactoSorb plating system provided adequate rigidity for stabilizing the osteotomized calvarial bone during surgery and maintained adequate rigidity after surgery during the bone healing period before absorption. This plating system showed satisfactory results in pediatric craniofacial surgery patients.

Absorbable Implants↗

[An exceptional form of cranio-facial fibrous dysplasia: fibrous dysplasia of middle turbinate].

Fibrous dysplasia is a rare bone disease that can be misdiagnosed because of the slowness of its symptoms. Although it is a benign disease its clinical behaviour can be severe, with possibility of local growth and malignant degeneration into osteosarcoma. An uncommon case of fibrous dysplasia is reported, affecting only the middle turbinate. Diagnostic and therapeutic problems are discussed.

Endoscopy↗